Renal Function Improves Mortality Risk Assessment in Pulmonary Arterial Hypertension - Pulmonology Advisor

Renal Function Improves Mortality Risk Assessment in Pulmonary Arterial Hypertension - Pulmonology Advisor
I Need to Be Understood, Not Fixed - Pulmonary Hypertension News
Meditating on the Power of Words One Sleepless Night - Pulmonary Hypertension News

Posted: 26 May 2020 08:30 AM PDT

In patients with pulmonary arterial hypertension (PAH), renal function is important for determining mortality risk and improves stratification of risk, according to the results of a retrospective study published in The Journal of Heart and Lung Transplantation.

Renal disease is a critical prognostic marker across many cardiovascular disease states and is prevalent in up to 36% to 47% of patients with PAH. However, despite the importance of renal function in patients with PAH, it has not been formally incorporated into many contemporary PAH risk assessment tools. Thus, researchers sought to compare contemporary risk assessment tools and determine the prognostic significance of risk parameters of kidney function and whether they can further improve risk prediction, particularly for those patients considered at intermediate risk.

A total of 211 treatment naive patients with PAH were categorized as low, intermediate, or high risk according to the European Society of Cardiology (ESC)/European Respiratory Society (ERS) 2015 guidelines, the French Pulmonary Hypertension Registry 2015 strategy, and the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) 1.0 and 2.0 risk calculators. The primary end point was transplant-free survival, which was a median of 7.09 years in the patient population.

The researchers observed little agreement between ESC- and REVEAL-based estimates. It was found that both ESC and REVEAL algorithms stratified transplant-free survival risk, with the REVEAL score providing superior discrimination (P =.004). Both renal function at diagnosis (P <.0001) and change in renal function at 6 months (P <.0001) were identified as novel risk parameters and were used to reclassify some patients from intermediate risk to low or high risk.

The authors concluded, "Our work highlights key limitations of the ESC/ERS-based risk assessment, and suggests that incorporating measures of kidney function are important strategies moving forward."

Disclosure: Several study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of authors' disclosures.

Reference

Zelt JGE, Hossain A, Sun LY, et al. Incorporation of renal function in mortality risk assessment for pulmonary arterial hypertension [published online April 5, 2020]. J Heart Lung Transplant. doi:10.1016/j.healun.2020.03.026

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PH Pulmonary Hypertension

I Need to Be Understood, Not Fixed - Pulmonary Hypertension News

Posted: 15 May 2020 12:00 AM PDT

When a new doctor enters my hospital room and asks for a brief medical history, the best I can offer is a 15-minute overview of the last few years of my life.

Realistically, for a proper understanding of my medical condition, my story should start at the beginning. My rare disease didn't just happen. I was born with a genetic condition called Currarino syndrome that ultimately affected almost every system of my body.

As I lay on a hospital bed two days ago having my port removed because of an infection, the surgeon chatted with me about my medical history and past surgeries. I expected her to say something similar to what everyone else says after I tell my story, such as, "Wow, you're so young to have all that going on."

But after listening to my story, the surgeon said something that made me realize what I seek from other care providers and medical professionals. She said that when she has a patient with a chronic illness, she can't cure them, but she can try to understand them.

The surgeon was able to fix me at my bedside by pulling out my port and draining my infection. But she couldn't fix why I had the port in the first place. I am sure that is frustrating for doctors because they want to be able to fix what is wrong. The doctors that I have had truly want to make me better. With chronic illness, though, I have days when things are better and days when I can barely get out of bed.

Having a care team that tries to understand is one of the greatest gifts. They aren't listening to my 10-minute speech about the last few years to respond with, "I can fix that." They listen to understand the complexity of my diagnosis. They try to learn more about what has worked and what hasn't to treat me the best they can. When my conditions worsen, there are medications that can make the pain better and procedures that can correct a piece of the puzzle, but none will make it go away.

For years, I have asked myself why this was happening. I was confused about why I wasn't getting better. I didn't understand why surgery didn't seem to correct the problem. I searched for tests, treatments, procedures, and a cure that didn't exist. I desperately wanted to help myself but didn't know how. For a while, it seemed like I would receive the same answer repeatedly and I didn't want to accept it. Doctors were telling me, "This is just how things are."

When I had genetic testing, the results brought me clarity, and I learned the various conditions that could result from having a mutated PCSK5 gene. The results shared by the geneticist and the rest of my team of doctors who have treated me since childhood helped me to accept that "this is how it is." To reach acceptance, I had to understand it for myself. But reaching that level of acceptance and understanding isn't easy.

Although I will never stop searching for a cure for future generations and advocating for improved outcomes and screening for prompt diagnosis, I have accepted that my chronic health conditions are a result of my rare disease. It isn't something that taking a medication or following a treatment can "fix." I can do everything possible to understand what is happening with my body to relieve my anxiety, but I can't stop any of it.

The surgeon helped me realize that just because medical professionals can't fix what is going on either, as frustrating as that may be, everyone can do their part in trying to understand.

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Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Hypertension News or its parent company, BioNews Services, and are intended to spark discussion about issues pertaining to pulmonary hypertension.

I am a 27 year old from the smallest state in the US, Rhode Island. I manage multiple chronic conditions, some are visible illnesses thanks to my oxygen I carry around, but most are invisible illnesses. I hope my posts "Recharged and Rewired" will show those reading that just because I need oxygen charged daily and my body is wired a little differently, doesn't mean I can't be the best version of myself every day!

Meditating on the Power of Words One Sleepless Night - Pulmonary Hypertension News

Posted: 04 May 2020 12:00 AM PDT

Lately, I have been reflecting on the power of words. It started one sleepless night, as I wondered how often I have thought or spoken the words pulmonary hypertension since my son's diagnosis. Even after his heart and lung transplant relieved him of that dreadful condition, not a day has gone by without PH crossing my mind or lips.

I cannot and should not stop thinking about the lives PH has taken and the growing numbers of children and adults fighting this illness. PH is more than a diagnosis or a disease. It is a silent stalker, thief, and murderer that will forever haunt the recesses of my mind.

Another word that has bothered me is "idiopathic." Personally, I think it is an ugly-sounding word. Medical dictionaries define it as a symptom or syndrome that appears without apparent cause. My son was diagnosed with PH when he was 8. Despite numerous tests, my seemingly healthy son was experiencing symptoms that could not be linked to any medication, coexisting condition, environmental influence, or genetics. His frustratingly final diagnosis was idiopathic pulmonary arterial hypertension.

This consumed my thoughts for years. It was inconceivable how he suddenly became symptomatic of an incurable and life-threatening condition. I spent many nights dreaming up theories to what caused his PH. I was worried that my son's illness was somehow my fault. I desperately wanted proof that it was caused by something beyond my control.

Through prayers, reflection, and deep discussions with my husband, I managed to heal my mind from these dark thoughts. All those sleepless nights trying to find a reason turned out to be a search for an answer that was not there. Ultimately, even if I knew the cause, it would not change the outcome. My attention was better served by helping my son fight PH than dwelling on the reason he had it in the first place.

As it turned out, his PH being idiopathic was a blessing. The myriad tests searching for a cause helped rule out conditions that could have made him ineligible for transplant. When that was explained, I decided that maybe not knowing was not so bad after all.

This brings me to the word "blessing," which has multiple uplifting meanings. Many believe it to mean God's favor and protection, and it can also be a prayer. It describes something that you feel deeply grateful for or refers to a person's sanction and support.

I think it is used too often, without thought or proper respect. It is a very personal word that I believe should be spoken with care. Someone I barely knew shared how blessed she felt having a healthy family when my son was so sick. I realize she meant well, and yes, she was blessed, but her use of the word made me wonder if it implied that my family was cursed.

Feeling angry and upset, I stewed over it for days until I had an epiphany. Not only am I blessed, but throughout my son's illness and transplant experiences, I have become deeply aware of just how blessed I am.

Throughout the long ordeal of diagnosis, treatment, transplant, and recovery, I found myself relying on several more words. Beautiful words like "faith," "hope," "love," "family," and "friendship." These words are life to me. Without them, I never could have found strength during struggle, happiness despite tears, and peace amid chaos.

When things have looked bleak, faith and hope have been the light in the dark guiding me to better days. I have been strengthened by prayer, and when too weak to find the words, others have prayed on my behalf. The love of family and friends has always been the foundation of my life. Without them I would have crumbled long ago.

I have felt peace over the years through the prayerful blessings of others. For example, there are no words that can describe how comforting it was to know that so many were praying for my son and our family on the day of his transplant. I honestly could feel our strength growing from the multitude of people praying for us.

Since then, I have made certain that I honor the words, "I will pray for you." It is not just something I say for lack of words, but rather a conscious commitment to doing it.

The next time you have trouble sleeping, focus on one word that has special meaning in your heart. Be mindful of how you take that word for granted and try to use it more wisely. If the word hurts, think about how you can take away its power. Consider how that one word has possibly led you to words that bring you peace, so that you can sleep.

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